PRNP and Creutzfeldt Jacob disease: These include the ki-3F4-FFI mice that harbour the D177N mutation, equivalent to the human mutation responsible for FFI [43] and the D178N Tg(FFI) also reproducing FFI phenotype [14], the Tg(A116V) mice mimicking A117V GSS [84], the TgMHuME199K mimicking E200K genetic CJD [30], the 113LBoPrP-Tg mice that incorporated the P113L substitution, homologous to the P102L mutation causing GSS in humans in a bovine PRNP transgenic mouse model [70], the P102L mutation of GSS in the murine prnp [68] or the 117VVTg30 mice also mimicking A117V GSS [6].